Steroid-responsive Devic’s variant in Sjogren’s syndrome

Sjogren’s syndrome is a chronic autoimmune disorder characterized by mononuclear infiltrates in the exocrine glands and various complications involving the peripheral and central nervous systems.1 Optic neuropathy is associated with this disease far less often than it is with other autoimmune vasculitic disorders such as systemic lupus erythematosus (SLE) and giant cell arteritis,2,3 and its mechanism has not been determined. We describe a patient with serologic and biopsy-confirmed Sjogren’s syndrome who had both a corticosteroid-responsive myelopathy and later an optic neuropathy. Additionally, a brain CT showed a swollen intraorbital optic nerve. At 58 years of age, a woman was admitted to the hospital because of the rapid onset of a visual field defect in her left eye. She had a history of a dry sensation in her mouth from her childhood. At age 51, she developed weakness in her arms and legs, had difficulty in walking, and was first admitted to the hospital. Neurologic examination on admission showed tetraparesis, sensory disturbance below the Th6 …